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Hayden, one of my four quadruplets, has an unknown progressive
syndrome that is slowly devastating his body. This is the story of how
we have chosen for our family to live life: preparing for the worst
while enjoying our life together.

Hayden's Story

After an intra-uterine insemination procedure,
we were surprised to find out that all four ovums developed into
embryos. But even early on we knew something was different about one:
it was far smaller than the other three. By 20 weeks, we had named each
baby and knew where each one was positioned. We named that little
fighter Hayden.

The
quadruplets were born after a relatively uncomplicated pregnancy at
32.4 weeks gestation. Hayden was the tiniest at 2 lbs 15 oz, but aside
from jaundice, he was a healthy preemie breathing on his own just like
his siblings: Peyton, Adrien, and Makenna.

Hayden's fight through typical preemie health
issues was more significant as compared to his siblings. He took longer
to put on weight, maintain his temperature, and nipple a bottle, but
after 31 days he graduated from the NICU and was reunited with his
sibling who had come home some 10-12 days before. Fast forward through
48 daily diaper changes and 24 bottles of pumped breast milk, and we
began to realize the gap in Hayden's development and his siblings was
becoming greater. We were referred to a neurologist, got lots of
testing including an MRI, and then got a devastating blow. It appeared
that the gray matter in Hayden's brain was shrinking. His neurologist
didn't know if it would stop shrinking or what the cause was, since he
had never seen anything like it.

Life with four babies was busy, and adding therapies and
specialists to the mix made it even more so. Over time, Hayden's
development remained static and severely delayed, but his health
declined. He started having seizures, silent reflux, constipation and eventually respiratory issues. Hospital stays became more frequent
with pneumonias, testing, and surgeries.

Making Tough
Decisions

With each
pneumonia infection, most often from aspiration, we had to brace
ourselves for a possible end to Hayden's life. At some point we learned
that it really wouldn't be the brain atrophy that killed Hayden, but
most likely an infection such as pneumonia. In many hospitalizations he was intubated, and
we were asked to consider a tracheotomy. This idea was presented many
times with increasing pneumonias, severe reflux, and floppy airways. At
some point during a routine follow-up with the pulmonologist, he
presented the idea that both my husband and I needed to have a serious
discussion to consider not only a tracheotomy, but also any life-sustaining
ideas. He suggested doing this while Hayden was acutely healthy, as the implications and
decisions have a different feel and may not be as well thought out when
in crisis.

We had
to be honest and consider how a tracheotomy would impact the lives of
both Hayden and our other children. It was our opinion that Hayden's
quality of life would be greatly diminished with the tracheotomy. To
add more artificial means of support that could lead to even more pain
and infections seemed to equate in our minds as more suffering. In
addition, we didn't have any skilled nursing support, and to add on
tracheotomy care to the already round-the-clock complexities of his care
would be overwhelming for our family. It is complete mayhem trying to
meet the individual needs of each child as it is, and his siblings were
already unintentionally ignored at times.

After much discussion and fact gathering, we
decided not to proceed with a tracheotomy. Physicians reassured us that
no matter what we decided, there really was no right answer, and they
would support us in whatever choice we made.

Just after the new year of 2009, Hayden had
surgeries for a hiatal hernia, as well as a far-reaching alternative to a
tracheotomy, which was to remove about 50% of his epiglottis tissue,
since his airway was so floppy and causing apnea spells. Those
surgeries were the start of a rough journey. Hayden developed
life-threatening infections and bowel obstructions. After a total of 86
non-consecutive days in the PICU with sepsis, pseudomonas infection in
his lungs, delays and extubation failures, and multiple GI surgeries, it
was determined that he would benefit from continued nutrition given
intravenously. It was adding another artificial means to sustaining his
life, but specialists and parents alike assumed it would be temporary,
and so we agreed to it.

Therapeutic Support
Plan and DNR

During
one of the long PICU stays, we were approached about how far we wanted
to proceed in maintaining Hayden's life. We met with his team of
specialists, and had individual discussions with each physician in great
detail. Ultimately, we put in place a conservative Therapeutic Support
Plan, including a Do Not Resuscitate (DNR) order for both the hospital
setting and home.

How
did we come to this decision? We had to consider the impact not only
on Hayden, but also on our entire family. We had to examine his level
of functioning and need for artificial life support, as well as consider
the severity of his disease and the unknown nature of its progression.

We also accepted that after countless blood,
urine, spinal and tissue samples being analyzed across multiple labs
with no diagnosis, that we may never know the name of a syndrome or have
any causative answers. We realized that having a name for his syndrome
doesn't equate a cure. The hard reality is that brain cells are not
replaceable, which is needed to be able to slow down or stop
progression. We still search, although not as passionately or as
far-reaching, for an answer, not for Hayden, but for his siblings and
their future children. It is possible that with an autopsy answers can be
found.

My husband
and I shared similar perspectives, which helped us to be honest with
each other and ourselves. We decided that with all that Hayden has
going on, it would stand to reason that no heroic measures should be
started. However, we would sustain his condition at a reasonable
level. In terms of quality of life, we would rather limit treatment than make the decision to remove treatments. Our plan includes
the following:

No
CPR

No inotropic or
heart-stimulating agents

No intubation

No mechanical ventilation

No dialysis

Blood products can be used to treat an acute and/or easily
correctable condition

Supportive Care

Since
Hayden had a Therapeutic Support Plan in place as well as IV therapy for
nutrition and medications, it was suggested that he would benefit from
supportive care. The term supportive care encompasses both palliative
and hospice support.

It was explained as additional support so that we don't have to
see a physician or make an emergency room visit every time Hayden gets
sick. The terms palliative and hospice were not really used with this
particular company, preferring the term Supportive Care. The Supportive
Care agency has a nurse who comes out every other week to draw labs
and to check vitals. Soon, we started to get calls seeing if we were
interested in having hospice volunteers. My husband and I were taken
aback by that term--hospice--and started to wonder if the physicians
failed to tell us our son was dying. I asked the nurse the next time
she was out, and it was explained that Supportive Care encompasses
palliative and hospice support, but that does not mean that Hayden has
to have an expected death date.

We soon allowed these so called hospice volunteers into our
lives. A group of teens from a high school about 20 miles away come out
once or twice month for a couple of hours to play with the kids so that
my husband and I can get stuff done around the house or even just get
some quality time in with Hayden. The quads adore the "big kids."

Our Supportive Care also has available an
Expressive Therapist who uses play therapy as her medium to help the
children cope with the constant medical crises and to learn about
death. The therapist has a session with the kids every month, and has
introduced the concept of death through Marc Brown's book, When Dinosaurs Die. The kids can
tell you reasons why a living being might die and have heard of people
dying due to a bad illness, but have yet to make the connection that
their brother could die from a bad illness. They do know that he gets
sick easily and that's why they have to be careful about hand-washing
and avoiding a lot of facial contact with him when they have "sickie
germs."

Some of the projects in the therapy include finding ways
to honor their brother through craft making. Since the kids are really
into imaginative play and mimicking adult and child roles through play
with their baby dolls and other toys, the therapist builds onto this.
It is not uncommon to see the baby dolls with a discarded syringe,
extension tube, or even a saline eclipse taped to the doll's chest. In
the next couple of play therapy sessions, the therapist plans on using
doll play to explain that the doll is very sick and building upon this
to explain that the doll can no longer get help from medicine, nurses,
doctors and hospitals. Eventually, there will be a funeral for the
doll.

The quads
also get support through a therapy dog and his handler, the
grandmotherly hospice volunteer. The dog makes monthly house calls.
Sometimes just petting and loving this dog is all they need to calm
down.

Daily Decisions

The summer Hayden came home with Supportive Care in place, we
were never sure how much we should plan ahead. There were the simple
things such as buying clothes for the next season. If I buy both the
boys matching outfits, will only one be wearing it by the time it fits?
There are also more expensive decisions, such as getting home
modifications to our existing house, or building a new house with main
floor accommodations for Hayden. How far or how soon do we go into
this? Will he live long enough to benefit from this?

There were other decisions about Hayden's
involvement in the community. Do we keep him isolated so that he is
less exposed to potential viral/bacterial threats? Do we send him to
preschool? We decided if we have a limited amount of time with him that
we'd make the most of it. I think it's been the best decision, and
well worth the risks. I can't imagine sitting at home all the time and
not allowing him to experience the world with the time he has left. We
tried it in the past before surgeries, keeping him isolated from
activities and events so as not to get sick beforehand. Since mom and
dad are his full-time and round-the-clock nurses, it meant we often had
to break up the family. One parent would stay home with Hayden while
the other with the three active kids.

One day my husband Andrew and I were talking
about childhood memories, and with a basic understanding of child
development, we came to decide that if long-term memories are forming
within the third year of life, than we will make a conscious decision to
create memories for the quads that have Hayden as part of their life.
We set forth to have a summer full of adventures. As Hayden healed from
abdominal incisions from the GI surgeries and responded really well to
intravenous nutrition, he took off. Even though Hayden does not have
the motor skills, speech, or vision to experience the adventures in the
same way that his siblings do, we actively helped him to engage in the
activities by hand-over-hand assistance, or through sensory
exploration. He responded really well with lots of smiles, contentment,
and laughs.

As he responded, his siblings shared in the
excitement. They began to help him engage in his surroundings and
experiences. Some of the proudest moments were when one of the quads
would shout out, "Mom he smiled at me! He laughs when we are
laughing! He looked right at me! He turned his head when the
cat walked by!" His siblings adore him and often come running when he
is distressed. They know just the right tricks to provide comfort. At
four years old, the children have an amazing sense of compassion and
love for their brother. We take tons of pictures. We never know when
Hayden's brain will fail to send the signals to breathe, cough out
secretions, start seizing uncontrollably or whatever. I take at
least one picture of him everyday, since we never know if it will be the
last.

He did
indeed go to preschool and even rode the bus twice a week. He responded
really well, and it has been an incredible experience. Hayden has
really started to interact in his own way and made developmental
"inchstones." We couldn't be more proud of his progress. He shows
excitement around his siblings with lots of kicking and turning toward
them as they play from afar and directly with him. This is a boy who
lost his smiles and they have returned with great frequency and
intention. His laughter is an indescribable experience too. The school
has gone above and beyond in his special needs classroom. School
specialists have taught us so much more about our son, and truly enjoy
and have great affection for him. I am confident that with these
experiences that Hayden gets the best quality of life we can offer.

A Unique Approach
For Every Family and Situation

Today we face even more questions about his fragile life. It
appears on a chest X-ray that he has another hiatal hernia. Based on
Hayden's history and what we learned, he may not survive anesthesia
again if he needs surgery. He may not be successful with extubation
post-surgery. If we decide not to proceed with surgery, does this mean
that his GI system will push further up into his diaphragm
compromising his lung capacity and creating a slow suffocation? For
now, we wait until we can get some answers before getting consumed with
worry.Like other families in this situation, there
are often numerous compromises to be made when balancing between
treatment and quality of life. The burden of chronic illness and the
care it demands can exhaust a family physically, emotionally, and
financially. Thankfully, at this point we do not have the latter worry,
but that can certainly change if we need to pay for nurses to care for
Hayden as his illness progresses. To be perfectly honest, the demands
of life with three very rambunctious healthy children and one with a
chronic illness send my husband and me into an exhausted and often times
interrupted sleep. If you ask either one of us, though, we both will
tell you how proud and happy we are with this life we've created with
our perfect family.

My
perspective on how we've approached and are handling the gravity of
having a child with a life-threatening and progressive illness may not
be agreeable to others and that's OK. We've drawn upon the wonderful
experiences to find the strength to make the hard decisions proactively,
such as developing a DNR. My husband and I have to get real in our
thinking patterns to avoid superstitious or "wishful thinking" that can
often times lead to false hopes. Moreover, rather than putting our
heads in the sand, we accepted the gravity of the situation.

Every situation is unique, but what I hope to
pass on is how we developed a pragmatic approach based on the love we
have for him and his siblings. In this family, we have actively engaged
in memory creation and family togetherness through experiences and
activities that allow us to take advantage of the time we have left.

Jamie and her husband Andrew have been married for 10 years and were blessed with quadruplets 4 1/2 years ago. Before the quads were born, she had a brief stint in social work before becoming a special education teacher, while earning her Masters Degree in Education. That background has been a huge asset in finding resources and understanding Hayden's needs. Hayden is truly the hero in his family's eyes.

Jamie regularly updates Hayden's Caringbridge page with the good, the bad, and sometimes the downright funny antics of life with four 4-year-olds. Follow along at www.caringbridge.org/visit/haydenbarnes

Copyright 2010 Complex Child E-Magazine. All Rights Reserved.

The information on these pages is not a substitute for appropriate medical care. Please contact your child's physicians before making any changes in your child's care. Complex Child is for research purposes only and does not constitute medical advice.